2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders

William J. Groh, Deepak Bhakta, Gordon F. Tomaselli, Ryan G. Aleong, Ricardo Alkmim Teixeira, Anthony Amato, Samuel J. Asirvatham, Yong Mei Cha, Domenico Corrado, Denis Duboc, Zachary D. Goldberger, Minoru Horie, Joseph E. Hornyak, John Lynn Jefferies, Stefan Kääb, Jonathan M. Kalman, Naomi J. Kertesz, Neal K. Lakdawala, Pier D. Lambiase, Steven A. LubitzHugh J. McMillan, Elizabeth M. McNally, Margherita Milone, Narayanan Namboodiri, Saman Nazarian, Kristen K. Patton, Vincenzo Russo, Frederic Sacher, Pasquale Santangeli, Win Kuang Shen, Dario C. Sobral Filho, Bruce S. Stambler, Claudia Stöllberger, Karim Wahbi, Xander H.T. Wehrens, Menachem Mendel Weiner, Matthew T. Wheeler, Katja Zeppenfeld

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. End-of-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs.

Original languageEnglish
Pages (from-to)e61-e120
JournalHeart Rhythm
Issue number10
StatePublished - Oct 2022


  • Atrial fibrillation
  • Atrioventricular block
  • Cardiomyopathy
  • ICD
  • Implantable cardioverter-defibrillator
  • Muscular dystrophy
  • Myotonic dystrophy
  • Pacemaker
  • Sudden death
  • Ventricular fibrillation
  • Ventricular tachycardia


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